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Carnitine palmitoyltransferase I

Index Carnitine palmitoyltransferase I

Carnitine palmitoyltransferase I (CPT1) also known as carnitine acyltransferase I, CPTI, CAT1, CoA:carnitine acyl transferase (CCAT), or palmitoylCoA transferase I, is a mitochondrial enzyme responsible for the formation of acyl carnitines by catalyzing the transfer of the acyl group of a long-chain fatty acyl-CoA from coenzyme A to l-carnitine. [1]

49 relations: Acetyl-CoA, Acetyl-CoA carboxylase, Beta oxidation, Binding site, Brown adipose tissue, C-terminus, Carnitine, Carnitine O-acetyltransferase, Carnitine O-octanoyltransferase, Carnitine palmitoyltransferase I deficiency, Carnitine palmitoyltransferase II, Catalysis, Catalytic triad, Coenzyme A, Competitive inhibition, Covalent bond, Cytosol, Diabetes mellitus, Diabetes mellitus type 2, Diffusion, Enzyme, Fatty acid, Fatty acid metabolism, Hepatic encephalopathy, Histidine, Hyperglycemia, Hyperinsulinemia, In silico, Inner mitochondrial membrane, Insulin resistance, Integral membrane protein, Knockout mouse, Malonate, Malonyl-CoA, Mitochondrial matrix, Mitochondrion, N-terminus, Oxyanion, Palmitoyl-CoA, Palmitoylcarnitine, Peptide, Protein isoform, Rate-determining step, Residue (chemistry), Serine, Thioester, Translocase, Wild type, X-ray crystallography.

Acetyl-CoA

Acetyl-CoA (acetyl coenzyme A) is a molecule that participates in many biochemical reactions in protein, carbohydrate and lipid metabolism.

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Acetyl-CoA carboxylase

Acetyl-CoA carboxylase (ACC) is a biotin-dependent enzyme that catalyzes the irreversible carboxylation of acetyl-CoA to produce malonyl-CoA through its two catalytic activities, biotin carboxylase (BC) and carboxyltransferase (CT).

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Beta oxidation

In biochemistry and metabolism, beta-oxidation is the catabolic process by which fatty acid molecules are broken down in the cytosol in prokaryotes and in the mitochondria in eukaryotes to generate acetyl-CoA, which enters the citric acid cycle, and NADH and FADH2, which are co-enzymes used in the electron transport chain.

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Binding site

In biochemistry, a binding site is a region on a protein or piece of DNA or RNA to which ligands (specific molecules and/or ions) may form a chemical bond.

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Brown adipose tissue

Brown adipose tissue (BAT) or brown fat makes up the adipose organ together with white adipose tissue (or white fat).

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C-terminus

The C-terminus (also known as the carboxyl-terminus, carboxy-terminus, C-terminal tail, C-terminal end, or COOH-terminus) is the end of an amino acid chain (protein or polypeptide), terminated by a free carboxyl group (-COOH).

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Carnitine

Carnitine (β-hydroxy-γ-N-trimethylaminobutyric acid, 3-hydroxy-4-N,N,N- trimethylaminobutyrate) is a quaternary ammonium compound involved in metabolism in most mammals, plants and some bacteria. Carnitine may exist in two isomers, labeled D-carnitine and L-carnitine, as they are optically active. At room temperature, pure carnitine is a white powder, and a water-soluble zwitterion with low toxicity. Carnitine only exists in animals as the L-enantiomer, and D-carnitine is toxic because it inhibits the activity of L-carnitine. Carnitine, derived from an amino acid, is found in nearly all organisms and animal tissue. Carnitine is the generic expression for a number of compounds that include L-carnitine, acetyl-L-carnitine, and propionyl-L-carnitine. It is most accumulated in cardiac and skeletal muscles as it accounts for 0.1% of its dry matter. It was first derived from meat extracts in 1905, therefore the name carnitine is derived from Latin "carnus" or flesh. The body synthesizes enough carnitine from lysine side chains to keep up with the needs of energy production in the body as carnitine acts as a transporter of long-chain fatty acids into the mitochondria to be oxidized and produce energy. Some individuals with genetic or medical disorders (like preterm infants) cannot make enough, so this makes carnitine a conditionally essential nutrient for them.

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Carnitine O-acetyltransferase

Carnitine O-acetyltransferase also called carnitine acetyltransferase (CRAT, or CAT) is an enzyme that encoded by the CRAT gene that catalyzes the chemical reaction where the acetyl group displaces the hydrogen atom in the central hydroxyl group of carnitine.

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Carnitine O-octanoyltransferase

In enzymology, a carnitine O-octanoyltransferase is an enzyme that catalyzes the chemical reaction Thus, the two substrates of this enzyme are octanoyl-CoA and L-carnitine, whereas its two products are CoA and L-octanoylcarnitine.

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Carnitine palmitoyltransferase I deficiency

Carnitine palmitoyltransferase I deficiency is a rare metabolic disorder that prevents the body from converting certain fats called long-chain fatty acids into energy, particularly during periods without food.

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Carnitine palmitoyltransferase II

Carnitine O-palmitoyltransferase 2, mitochondrial is an enzyme that in humans is encoded by the CPT2 gene.

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Catalysis

Catalysis is the increase in the rate of a chemical reaction due to the participation of an additional substance called a catalysthttp://goldbook.iupac.org/C00876.html, which is not consumed in the catalyzed reaction and can continue to act repeatedly.

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Catalytic triad

A catalytic triad is a set of three coordinated amino acids that can be found in the active site of some enzymes.

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Coenzyme A

Coenzyme A (CoA,SCoA,CoASH) is a coenzyme, notable for its role in the synthesis and oxidation of fatty acids, and the oxidation of pyruvate in the citric acid cycle.

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Competitive inhibition

Competitive inhibition is a form of enzyme inhibition where binding of an inhibitor prevents binding of the target molecule of the enzyme, also known as the substrate.

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Covalent bond

A covalent bond, also called a molecular bond, is a chemical bond that involves the sharing of electron pairs between atoms.

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Cytosol

The cytosol, also known as intracellular fluid (ICF) or cytoplasmic matrix, is the liquid found inside cells.

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Diabetes mellitus

Diabetes mellitus (DM), commonly referred to as diabetes, is a group of metabolic disorders in which there are high blood sugar levels over a prolonged period.

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Diabetes mellitus type 2

Diabetes mellitus type 2 (also known as type 2 diabetes) is a long-term metabolic disorder that is characterized by high blood sugar, insulin resistance, and relative lack of insulin.

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Diffusion

Diffusion is the net movement of molecules or atoms from a region of high concentration (or high chemical potential) to a region of low concentration (or low chemical potential) as a result of random motion of the molecules or atoms.

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Enzyme

Enzymes are macromolecular biological catalysts.

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Fatty acid

In chemistry, particularly in biochemistry, a fatty acid is a carboxylic acid with a long aliphatic chain, which is either saturated or unsaturated.

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Fatty acid metabolism

Fatty acid metabolism consists of catabolic processes that generate energy, and anabolic processes that create biologically important molecules (triglycerides, phospholipids, second messengers, local hormones and ketone bodies).

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Hepatic encephalopathy

Hepatic encephalopathy (HE) is an altered level of consciousness as a result of liver failure.

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Histidine

Histidine (symbol His or H) is an α-amino acid that is used in the biosynthesis of proteins.

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Hyperglycemia

Hyperglycemia, or high blood sugar (also spelled hyperglycaemia or hyperglycæmia) is a condition in which an excessive amount of glucose circulates in the blood plasma.

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Hyperinsulinemia

Hyperinsulinemia, or hyperinsulinaemia is a condition in which there are excess levels of insulin circulating in the blood relative to the level of glucose.

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In silico

In silico (literally cod Latin for "in silicon", alluding to the mass use of silicon for semiconductor computer chips) is an expression used to mean "performed on computer or via computer simulation." The phrase was coined in 1989 as an allusion to the Latin phrases in vivo, in vitro, and in situ, which are commonly used in biology (see also systems biology) and refer to experiments done in living organisms, outside living organisms, and where they are found in nature, respectively.

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Inner mitochondrial membrane

The inner mitochondrial membrane (IMM) is the mitochondrial membrane which separates the mitochondrial matrix from the intermembrane space.

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Insulin resistance

Insulin resistance (IR) is a pathological condition in which cells fail to respond normally to the hormone insulin.

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Integral membrane protein

An integral membrane protein (IMP) is a type of membrane protein that is permanently attached to the biological membrane.

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Knockout mouse

A knockout mouse or knock-out mouse is a genetically modified mouse (Mus musculus) in which researchers have inactivated, or "knocked out", an existing gene by replacing it or disrupting it with an artificial piece of DNA.

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Malonate

The malonate or propanedioate ion is CH2(COO)22− (malonic acid minus two hydrogen ions).

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Malonyl-CoA

Malonyl-CoA is a coenzyme A derivative of malonic acid.

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Mitochondrial matrix

In the mitochondrion, the matrix is the space within the inner membrane.

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Mitochondrion

The mitochondrion (plural mitochondria) is a double-membrane-bound organelle found in most eukaryotic organisms.

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N-terminus

The N-terminus (also known as the amino-terminus, NH2-terminus, N-terminal end or amine-terminus) is the start of a protein or polypeptide referring to the free amine group (-NH2) located at the end of a polypeptide.

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Oxyanion

An oxyanion, or oxoanion, is an ion with the generic formula (where A represents a chemical element and O represents an oxygen atom).

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Palmitoyl-CoA

Palmitoyl-CoA is an acyl-CoA thioester used in the biosynthesis of sphingosine: Palmitoyl-CoA is part of the carnitine shuttle system, which transports other fatty acyl-CoA molecules into the mitochondria for β-oxidation.

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Palmitoylcarnitine

Palmitoylcarnitine is an ester derivative of carnitine involved in the metabolism of fatty acids.

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Peptide

Peptides (from Gr.: πεπτός, peptós "digested"; derived from πέσσειν, péssein "to digest") are short chains of amino acid monomers linked by peptide (amide) bonds.

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Protein isoform

A protein isoform, or "protein variant" is a member of a set of highly similar proteins that originate from a single gene or gene family and are the result of genetic differences.

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Rate-determining step

In chemical kinetics, the overall rate of a reaction is often approximately determined by the slowest step, known as the rate-determining step (RDS) or rate-limiting step.

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Residue (chemistry)

In chemistry residue is whatever remains or acts as a contaminant after a given class of events.

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Serine

Serine (symbol Ser or S) is an ɑ-amino acid that is used in the biosynthesis of proteins.

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Thioester

In chemistry thioesters are compounds with the functional group R–S–CO–R'.

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Translocase

Translocase is a general term for a protein that assists in moving another molecule, usually across a membrane.

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Wild type

Wild type (WT) refers to the phenotype of the typical form of a species as it occurs in nature.

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X-ray crystallography

X-ray crystallography is a technique used for determining the atomic and molecular structure of a crystal, in which the crystalline atoms cause a beam of incident X-rays to diffract into many specific directions.

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Redirects here:

CPT1, CPT1A, CPT1B, CPT1C, Carnitine palmitoyltransferase i, Carnitine palmitoyltransferase-1, Carnitine-palmitoyl transferase I, Mitochondrial carnitine palmitoyltransferase-1.

References

[1] https://en.wikipedia.org/wiki/Carnitine_palmitoyltransferase_I

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